Hypoplastic Left Heart Syndrome
- Although surgical intervention has become the medical standard, supportive measures are sometimes offered, especially when multiple noncardiac congenital anomalies exist or when severe multiorgan system damage is present.
- The preoperative goal is to balance the systemic and pulmonary circulations provided by the RV to a Qp/Qs (ratio of pulmonary to systemic blood flow) of ~1:1, usually achieved with a pulse oximetry measurement of 75%.
- Prostaglandin E1 infusion: 0.05–0.1 mcg/kg/min
- Aggressive treatment of metabolic acidosis with fluid boluses, bicarbonate, and/or tromethamine (THAM).
- 0.21 FiO2, goal PaO2 of 35–40 mm Hg
- Careful use of small amounts of inotropic agents (in cases of sepsis or RV failure). Aggressive use of inotropic agents (alpha effect) may worsen systemic perfusion.